Thrombotic thrombocytopenic purpura
| TTP - this is worth waking up the on call haematologist. | |
|---|---|
| Etiology | Autoimmune (40-70%), drug-related (e.g. clopidogrel), hereditary ADAMST13 mutations... see below |
| Clinical presentation | Fever, MAHA, purpuric rash, neuro symptoms, AKI Complications: |
| Pathogenesis | ADAMTS13 damage or inhibition → vWF not cleaved → microvascular thrombosis |
| Diagnostic investigations | Thrombocytopenia; ↑LDH, ↓ haptoglobins, clotting (normal). Exclude infection. Blood film: schistocytes |
| Management | Plasma exchange, FFP, rituximab |
etiologies
- Autoimmune haemolysis/Evans syndrome
- Disseminated intravascular coagulation
- Pregnancy‐associated e.g. HELLP (haemolysis, elevated liver enzymes and low platelets), eclampsia
- Drugs eg quinine, simvastatin, interferon, Calcineurin inhibitors
- Malignant hypertension
- Infections, typically viral (cytomegalovirus, adenovirus, Herpes simplex virus) or severe bacterial (meningococcus, pneumococcus), fungal
- Autoimmune disease (lupus nephritis, acute scleroderma)
- Vasculitis
- Haemolytic uraemic syndrome (diarrhoea positive/negative)
- Malignancy
- Catastrophic antiphospholipid syndrome